Discordance between clinical, physiological, and radiological measures in cystic fibrosis

There are several methods available to the clinician to assess severity of lung disease in cystic fibrosis (CF). Here we present a case where several of these modalities appeared to be discordant. As in this case, many assessments of respiratory status may be necessary to fully evaluate patients with CF. Spirometry may be insensitive to significant changes that are apparent both clinically and radiologically, but remains an important marker of short-term change, response to intervention, and prognosis. Chest computed tomography can provide valuable detailed information on the health of the lungs and is more sensitive than spirometry to the changes seen in CF. Longitudinal studies of subjects with disproportionately severe radiological changes may be important to assess whether they are more at risk of precipitous declines in pulmonary function.